PMID- 15366655
OWN - NLM
STAT- MEDLINE
DCOM- 20050222
LR  - 20161021
IS  - 0125-877X (Print)
IS  - 0125-877X (Linking)
VI  - 22
IP  - 1
DP  - 2004 Mar
TI  - Immunological and clinical features of pediatric patients with primary 
      hypogammaglobulinemia in Taiwan.
PG  - 25-31
AB  - We retrospectively reviewed the clinical and immunological features as well as 
      the outcome of children with a diagnosis of primary hypogammaglobulinemia, who 
      were treated at the National Taiwan University Hospital between 1984 and 2001. A 
      total of 33 patients were enrolled: seventeen patients with common variable 
      immunodeficiency (CVID), six patients with selective immunoglobulin deficiencies 
      (one subclass IgA and five IgG), four patients with severe combined 
      immunodeficiency (SCID), three patients with transient hypogammaglobulinemia of 
      infancy (THI) and three patients with X-linked (Bruton) agammaglobulinemia (XLA). 
      In addition to recurrent sinopulmonary infections and prolonged fever, allergic 
      diseases are noted in 76% of CVID patients and 100% of patients with selective 
      immunodeficiencies. Immunoglobulin levels were extremely low in XLA and decreased 
      in CVID patients. Three SCID patients had decreased mean absolute lymphocyte 
      counts of 290/mm3. Long-term complications included bronchiectasis in 2 XLA 
      patients, 2 CVID patients and 1 patient with selective immunodeficiency; short 
      stature in one of each XLA, SCID, and CVID patients respectively; poor school 
      performance in 2 SCID patients and 1 XLA patient; and hemolytic anemia in 1 CVID 
      patient. We concluded that in addition to a thorough physical examination, a 
      family history of early death from infection and past history of neonatal 
      hyperbilirubinemia, are crucial in evaluating a patient with suspicious primary 
      hypogammaglobulinemia. The associated symptoms of primary hypogammaglobulinemia, 
      such as recurrent sinopulmonary infections, prolonged fever and allergic 
      diseases, are also diagnostic clues. In the treatment of hypogammaglobulinemia, 
      early and regular high doses of Intravenous immunoglobulin (IVIG) supplement may 
      avoid the development or decrease the severity of bronchiectasis.
FAU - Wang, Ling-Jen
AU  - Wang LJ
AD  - Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.
FAU - Yang, Yao-Hsu
AU  - Yang YH
FAU - Lin, Yu-Tsan
AU  - Lin YT
FAU - Chiang, Bor-Luen
AU  - Chiang BL
LA  - eng
PT  - Journal Article
PL  - Thailand
TA  - Asian Pac J Allergy Immunol
JT  - Asian Pacific journal of allergy and immunology
JID - 8402034
SB  - IM
MH  - Agammaglobulinemia/complications/diagnosis/*immunology
MH  - Child, Preschool
MH  - Female
MH  - Humans
MH  - Immunologic Deficiency Syndromes/complications/diagnosis/*immunology
MH  - Infant
MH  - Male
MH  - Retrospective Studies
MH  - Taiwan
MH  - Treatment Outcome
EDAT- 2004/09/16 05:00
MHDA- 2005/02/23 09:00
CRDT- 2004/09/16 05:00
PHST- 2004/09/16 05:00 [pubmed]
PHST- 2005/02/23 09:00 [medline]
PHST- 2004/09/16 05:00 [entrez]
PST - ppublish
SO  - Asian Pac J Allergy Immunol. 2004 Mar;22(1):25-31.